腦下垂體腺瘤切除術後併發橋腦中心及橋腦外髓鞘溶解症：病例報告

橋腦中心髓鞘溶解症(central pontine myelinolysis, CPM)與橋腦外髓鞘溶解症(extrapontine myelinolysis, EPM)是同一疾病，其病理變化相同，但因病灶位置不同而有不同的臨床症狀。雖然至目前為止，對於橋腦中心及橋腦外髓鞘溶解症之病因及病理機轉仍未有定論，但快速矯正低血鈉被證實為造成橋腦中心及橋腦外髓鞘溶解症之一項主要原因，因此正確地矯正低血鈉，對於預防橋腦中心或橋腦外髓鞘溶解症之發生十分重要。腦下垂體腺瘤切除術後發生低血鈉之比率，過去報告自8.4%至38.8%，不過在文獻報告中，患者於術後發生有症狀之低血鈉情形，大多數均可在治療後康復，並且未留下神經學損傷，僅有三則病例報告患者在治療後，由於血鈉濃度升高的速率過快，而導致橋腦中心或橋腦外髓鞘溶解症之發生。本病例報告一病患，在接受腦下垂體腺瘤切除手術後，腦下垂體前葉功能減低，並出現了嚴重低血鈉之症狀，因此患者除接受鈉離子補充治療，同時也接受了甲狀腺素及類固醇(hydrocortisone)之激素補充。然而此病患經初步治療後，卻造成血鈉濃度的急遽上升，引發了橋腦中心及橋腦外之髓鞘溶解症，以精神及意識變化、眼肌麻痺、吞嚥障礙、四肢癱瘓等為主要表現。由於造成此病患發生低血鈉的主要原因，來自於腦下垂體前葉功能低下所導致之甲狀線及腎上腺功能低下，因此適當補充其所缺少的激素，才是矯正其低血鈉治本之道，給予額外的鈉離子補充治療，可能反而在色者甲狀腺及腎上腺功能逐漸恢復時，造成血鈉濃度篡升。因此，對於低血鈉的患者，應針對其引發低血鈉的主要病因給予正確的治療；倘若需給予鈉離子補充治療，則應密集地追蹤血鈉值並避免血鈉濃度快速改變，進而減少橋腦中心或橋腦外髓鞘溶解症及其相關併發症的發生。

關鍵字

橋腦中心髓鞘溶解症；橋腦外髓鞘溶解症；腦下垂體線瘤；低血鈉

並列摘要

Central pontine myelinolysis(CPM) and extrapontine myelinolysis(EPM), sharing the same pathology, are the same disease, although different symptoms and signs developed according to different lesion sites. The etiology and pathogenesis of CPM/EPM remain unclear, meanwhile rapid correction of the serum sodium level for hyponatremic patient has proven to be one of the major causes of CPM/EPM. Therefore an appropriate correction of the sodium level could prevent the development of CPM/EPM. The incidence of hyponatremia after surgical removal of pituitary adenoma reported varies from 8.4% to 38.8%. Most patients with symptomatic hyponatremia after the surgery recovered without neurological deficits. Only three cases have been reported to have central pontine or extrapontine myelinolysis due to the rapid elevation of serum sodium level after initial management. Our report presents a case who, after surgical removal of pituitary adenoma, suffered from severe hyponatremia secondary to anterior hypopituitarism. Replacement of thyroxin and hydrocortisone was applied in addition to salt replacement. However, the serum sodium level elevated abruptly, resulting in central pontine and extrapontine myelinolysis with the clinical manifestation of psychiatric disorder and conscious disturbance, ophthalmoparesis, dysphagia, and quadreparesis. Since hypothyroidism and adrenal gland insufficiency resulted from anterior hypopituitarism were considered the main causative factors of hyponatremia in this case, certain hormone replacements should correct the sodium level. The supplementation of salt might produce additional sodium loading thus inappropriately increasing the sodium level. We conclude that hyponatremia should be treated with respect to certain underlying diseases. Cautiously correcting the sodium imbalance and frequently following-up with measurements of the serum sodium level during sodium replacement therapy are essential to preventing CPM/EPM and the related complications.

並列關鍵字

central pontine myelinolysis ； extrapontine myelinolysis ； pituitary adenoma ； hyponatremia

國際替代計量

腦下垂體腺瘤切除術後併發橋腦中心及橋腦外髓鞘溶解症：病例報告

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